Second series by the Italian Association of Pediatric Hematology and Oncology of children and adolescents with intracranial ependymoma: an integrated molecular and clinical characterization with a long-term follow-up.
Maura MassiminoFrancesco BarrettaPiergiorgio ModenaHendrik WittSimone MinasiStefan M PfisterKristian W PajtlerManila AntonelliLorenza GandolaMaria Luisa GarrèDaniele BertinAngela MastronuzziMaurizio MascarinLucia QuagliettaElisabetta ViscardiIacopo SardiAntonio RuggieroBianca PolloAnnamaria BuccolieroLuna BoschettiElisabetta SchiavelloLuisa ChiappariniAlessandra ErbettaIsabella MorraMarco GessiVittoria DonofrioCarlo PatriarcaFelice GiangasperoPascal JohannFrancesca Romana ButtarelliPublished in: Neuro-oncology (2021)
Previously described prognostic factors were confirmed at 10-year follow-up. Late relapses occurred in 6.2% of patients. Specific molecular features may affect outcome: PFB patients had a very good prognosis; 1q gain and CDKN2A loss were associated with dissemination. To draw reliable conclusions, modern ependymoma trials need to combine diagnostics with molecular risk stratification and long-term follow-up.